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Multiple jaw cysts-unveiling the Gorlin-Goltz syndrome.

Manjima S, Naik Z, Keluskar V, Bagewadi A - Contemp Clin Dent (2015)

Bottom Line: Dentist plays a major role in the diagnosis of this disease due to the oral and maxillofacial manifestations of the syndrome.Odontogenic keratocysts in such syndromic patients will be multiple and extensive and in some cases results in cortical expansion and facial disfigurement.Thorough clinical examination and investigations prompt an early confirmation of the syndrome, which is very essential to avoid morbidity associated with the syndrome.

View Article: PubMed Central - PubMed

Affiliation: Department of Oral Medicine and Radiology, KLE VKIDS, Belgaum, Karnataka Belgaum, India.

ABSTRACT
Gorlin-Goltz syndrome or basal cell nevus syndrome is a comparatively rare syndrome characterized by basal cell nevi, odontogenic keratocysts, and skeletal anomalies. Diagnosis is based on the major and minor clinical and radiographic criteria. Dentist plays a major role in the diagnosis of this disease due to the oral and maxillofacial manifestations of the syndrome. In some cases, jaw cysts are diagnosed by routine radiographs advised by the dentists. Odontogenic keratocysts in such syndromic patients will be multiple and extensive and in some cases results in cortical expansion and facial disfigurement. Thorough clinical examination and investigations prompt an early confirmation of the syndrome, which is very essential to avoid morbidity associated with the syndrome. Here, we report a case of multiple odontogenic cysts in a 16-year-old patient which later was diagnosed as a case of Gorlin Goltz syndrome.

No MeSH data available.


Related in: MedlinePlus

Hyperchomatic palisaded basal layer and corrugated parakeratotic surface (H and E, ×10)
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Figure 7: Hyperchomatic palisaded basal layer and corrugated parakeratotic surface (H and E, ×10)

Mentions: An incisional biopsy of the swelling was advised. Histopathological examination of specimen revealed cystic epithelium composed of darkly stained basal cells arranged in a palisading pattern. Superficial layer was showing parakeratinization and corrugated appearance which was suggestive of odontogenic keratocyst [Figure 7].


Multiple jaw cysts-unveiling the Gorlin-Goltz syndrome.

Manjima S, Naik Z, Keluskar V, Bagewadi A - Contemp Clin Dent (2015)

Hyperchomatic palisaded basal layer and corrugated parakeratotic surface (H and E, ×10)
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4374303&req=5

Figure 7: Hyperchomatic palisaded basal layer and corrugated parakeratotic surface (H and E, ×10)
Mentions: An incisional biopsy of the swelling was advised. Histopathological examination of specimen revealed cystic epithelium composed of darkly stained basal cells arranged in a palisading pattern. Superficial layer was showing parakeratinization and corrugated appearance which was suggestive of odontogenic keratocyst [Figure 7].

Bottom Line: Dentist plays a major role in the diagnosis of this disease due to the oral and maxillofacial manifestations of the syndrome.Odontogenic keratocysts in such syndromic patients will be multiple and extensive and in some cases results in cortical expansion and facial disfigurement.Thorough clinical examination and investigations prompt an early confirmation of the syndrome, which is very essential to avoid morbidity associated with the syndrome.

View Article: PubMed Central - PubMed

Affiliation: Department of Oral Medicine and Radiology, KLE VKIDS, Belgaum, Karnataka Belgaum, India.

ABSTRACT
Gorlin-Goltz syndrome or basal cell nevus syndrome is a comparatively rare syndrome characterized by basal cell nevi, odontogenic keratocysts, and skeletal anomalies. Diagnosis is based on the major and minor clinical and radiographic criteria. Dentist plays a major role in the diagnosis of this disease due to the oral and maxillofacial manifestations of the syndrome. In some cases, jaw cysts are diagnosed by routine radiographs advised by the dentists. Odontogenic keratocysts in such syndromic patients will be multiple and extensive and in some cases results in cortical expansion and facial disfigurement. Thorough clinical examination and investigations prompt an early confirmation of the syndrome, which is very essential to avoid morbidity associated with the syndrome. Here, we report a case of multiple odontogenic cysts in a 16-year-old patient which later was diagnosed as a case of Gorlin Goltz syndrome.

No MeSH data available.


Related in: MedlinePlus